The classification of kidney tumors in adults expands rapidly with new categories recently incorporated. This will result in the modification of the current 2004 World Health Organization (WHO) classification of the adult renal epithelial neoplasms. Emphasis should be placed in defining risk groups categorized as malignant or benign tumors, including a category of tumors with low malignant potential to accommodate recently recognized categories with extremely good prognosis after surgery. Unusual tumors such as familial renal cell carcinoma (RCC), translocation RCC, renal cell carcinoma after neuroblastoma, tubular mucinous and spindle cell carcinoma, and mixed epithelial and stromal tumors are also presented. A number of recently described entities and morphologic variants of classical categories deserve recognition since they can be important in differential diagnosis. This review emphasizes clinical, pathological and genetic features defining renal epithelial tumors in adults.